A Case of Rosai-Dorfman Disease Involving the Unilateral Neck and the Abdominal Cavity

Rosai-Dorfman disease is a rare lymph node disorder characterized by abnormal proliferation of histiocytes and usually presents with bilateral cervical lymphadenopathy. This disease has been poorly understood by otolaryngologists because of its rarity. We report an unusual case of a 61-year-old woman with Rosai-Dorfman disease involving one side of the neck and the abdominal cavity, which distinguishes our case from previous reports. Her symptoms and image findings were consistent with that of a lymphoma. However, histopathological examination of the lymph node revealed emperipolesis, a characteristic feature of Rosai-Dorfman disease. After medical treatment with steroids for 2 weeks, the size of the lymph node decreased by 50%. Since the clinical course of Rosai-Dorfman disease varies from spontaneous remission to fatal outcomes, we believe that Rosai-Dorfman disease should be considered in the differential diagnosis of patients with cervical lymphadenopathy. (J Clinical Otolaryngol 2013;24:291-295)